乳头状肾癌的诊治（附32例报告及文献复习）

目的：提高乳头状。肾癌的诊治水平。方法：回顾性分析32例乳头状肾癌患者的临床资料。28例行开放性根治性肾切除术,3例行后腹腔镜下根治性肾切除术,1例行后腹腔镜下肾部分切除。结果：术后病理均为乳头状肾癌。采用2002年AJCC肾癌TNM病理分期：pT1oNoMo4例,pT1bNoMo14例,pT2oNoMo10例,pT2bNoMo1例,pTzbN·Mo1例,pT3oNoMo2例,病理分级：G121例,G210例,G31例。28例获16～30个月随访,平均20个月,2例死于心脏病,3例死于脑血管意外,1例局部复发,22例无瘤生存。结论：乳头状肾癌是一种具有特殊形态和表现的少见肾癌类型,部分。肾切除及根治性肾切除术是治疗乳头状。肾癌的首选方法,与其他类型肾细胞癌相比,乳头状肾癌预后较好。     

肾嫌色细胞癌21例临床分析及文献复习

目的 提高肾嫌色细胞癌的诊治水平和对此类型肾癌的认识.方法 回顾性分析21例肾嫌色细胞癌的临床资料.男11例,女10例.年龄27 ～ 85岁,平均52岁.11例行腹腔镜下肾癌根治术,8例行腹腔镜下肾部分切除术,2例行开放肾癌根治术.结果 术后病理证实为肾嫌色细胞癌.病理分期:pT1N0M0 13例,pT2N0M0 5例,pT3aN1 M0 2例,pT4N0M0 1例.Fuhrman病理分级:G1 6例,G2 14例,G31例.术后随访19例,时间3～36个月,平均17个月,1例死于心脏病,1例术后6个月局部复发,给予索拉非尼治疗2个月后肺部感染死亡,1例术后12个月后出现肺转移,给予索拉非尼治疗1个月后死亡,16例无瘤生存.结论 肾嫌色细胞癌是一种具有特殊形态的少见肾癌类型.肾根治性切除术是治疗肾嫌色细胞癌的首选方法.与同期、同级的其他类型肾癌相比,肾嫌色细胞癌预后较好.     

肾嫌色细胞癌(附15例报告)

目的　提高肾嫌色细胞癌的诊治水平和对此类型肾癌的认识。　方法　回顾性分析15例肾嫌色细胞癌的临床资料。男 10例 ,女 5例。年龄 4 7～ 74岁 ,平均 5 7岁。均行根治性肾切除术。　结果　术后病理证实为肾嫌色细胞癌。病理分期 :pT1N0 M0 6例 ,pT2 N0 M0 5例 ,pT3bN0 M0 2例 ,pT1N2 M0 1例 ,pT2 N2 M0 1例。病理分级 :G2 10例 ,G3 5例。 11例获随访 ,随访 2～ 31个月 ,平均19个月 ,1例死于心脏病 ,1例局部复发 ,9例无瘤生存。　结论　肾嫌色细胞癌是一种具有特殊形态的少见肾癌类型。肾根治性切除术是治疗肾嫌色细胞癌的首选方法。与同期、同级的其他类型肾癌相比 ,肾嫌色细胞癌预后较好。     

多房囊性肾细胞癌手术治疗的预后特点分析

目的探讨多房囊性肾细胞癌患者手术治疗的预后特点。方法回顾性分析482例肾癌患者中22例多房囊性肾细胞癌患者资料，分析其手术治疗的预后特点。多房囊性肾细胞癌占同期肾癌病例的4．56％，男女比例为2．67：1．00，年龄32～74岁，平均47岁。结果22例患者中行肾癌根治术18例，行肾部分切除术4例。肿瘤直径1．8～11．0cm，平均4．8cm。其中透明细胞癌21例，透明细胞癌与颗粒细胞癌混合型1例；pT1N0M019例，pT2N0M02例；pT3bN0M01例；G15例，G217例。失访2例，20例获随访，随访时间9～56个月，平均27．6个月。其中1例因肝硬化、上消化道出血死亡，无瘤存活21例。结论多房囊性肾癌是肾癌的一种特殊类型，多为肾偶发癌，病理分期分级低，预后与肿瘤大小无关，手术治疗效果满意，预后佳。     

双侧肾细胞癌21例报告

目的 探讨双侧肾细胞癌手术治疗的疗效。方法 1990年1月至2005年1月收治双侧肾细胞癌患者21例。其中同时性10例，异时性11例。肿瘤位于肾上极15枚，中极19枚，下极20枚；左肾28枚，右肾26枚。肿瘤直径0．5～16．0cm，平均4．5cm。2例伴下腔静脉癌栓。17例经手术治疗。6例同时性肾癌中，5例行双侧一期手术，1例行分期手术。其中1例行双肾部分切除术；1例行双肾部分切除及左肾上腺切除术；3例行一侧肾癌根治术及对侧肾部分切除术；1例行一侧肾癌根治术，对侧肾部分切除术及下腔静脉切开取癌栓术。11例异时性肾癌均分期手术。其中3例行双肾部分切除术；1例行一侧肾癌根治术，对侧肾肿瘤剜出术；1例行一侧肾癌根治术，对侧肾部分切除术加下腔静脉切开取癌栓术；6例行一侧肾癌根治术，对侧肾部分切除术。未行手术治疗4例．其中行介入治疗1例，生物学治疗1例，保守治疗2例。结果 21例随访3个月～24年，平均3．4年。17例手术治疗者中，13例未见肿瘤复发和转移，术后肾功能均可；1例术后10个月出现左上肺转移，已带瘤存活9个月；1例术后1年出现残肾肿瘤复发，经生物学和中药治疗，已带瘤存活5个月；2例死于肿瘤转移。4例未手术者中，1例10个月后死于胰腺转移，1例4个月后死于肾衰竭，1例6个月后死于脑血管意外，1例伴腹膜后淋巴结转移者经化疗和生物学治疗，病情稳定，已带瘤存活7个月。异时性肾癌者的先发一侧行肾癌根治术，对侧肾出现肿瘤的时间为7个月～18年。结论 双侧肾癌并非手术禁忌，如果采取积极的治疗措施，仍可获得满意的手术效果。保肾手术是目前较为理想的治疗方法，如果保留足够的肾实质，患者仍可获得良好的生存状态。     

多房囊性肾细胞癌的临床特点及预后

目的评价多房囊性肾细胞癌的诊疗特点及预后。方法回顾性分析6例多房囊性肾细胞癌的临床特点、影像学表现、外科手术方法、病理特点,随访其预后。站果男3例,女3例,肿瘤最大直径3．0～7．5cm。CT示多囊性肿物,实性部位平扫CT值30～36Hu,皮质期88～130Hu,排泄期75～96Hu,为隔膜型增强型。腹腔镜肾部分切除术1例,根治性肾切除5例。病理TNM分期,pT1aN0M0 2例,pT1bN0M03例,pT2N0M01例,癌细胞核分级均为G1。中位随访24个月,随访期内未发现远处转移及肿瘤复发,无瘤存活100％。结论多房囊性肾细胞癌为一种特殊的病理类型,病理分期、分级低,预后良好,术前怀疑或术中证实为多房囊性肾细胞癌应考虑行腔镜保留肾单位的肾部分切除术。     

肾嫌色细胞癌29例临床分析简

目的 探讨肾嫌色细胞癌的临床特点,提高其临床诊治水平。方法 回顾性分析2006年6月至2012年4月期间收治的29例肾嫌色细胞癌患者的临床资料,包括临床表现、影像学检查、治疗方法、病理分期和随访等。结果 29例患者均缺乏典型的临床症状,术前影像学检查亦未发现特异性表现。16例行根治性肾切除术,其余13例行保留肾单位手术,病理分期： pT 1 N 0 M 0 27例,pT 2 N 0 M 0 2 例。29例患者均获得随访,随访13-72个月,平均（35.0±7.2）个月,29例均无瘤生存。结论 肾嫌色细胞癌是肾细胞癌的一个独立类型,CT和B超检查缺乏特异性表现,主要通过病理学进行诊断,但CT表现与常见的肾透明细胞癌有所不同,手术切除是治疗肾嫌色细胞癌的首选方法,其预后较好。     

肾集合管癌的临床特征分析

目的探讨肾集合管癌的临床病理特征及预后。方法回顾性研究2007年3月至2012年6月间收治的5例肾集合管癌患者的临床及病理资料并进行随访。结果5例肾集合管癌占同期肾癌的0．74％,男3例,女2例,平均年龄61岁。左肾4例,右肾1例。主要症状为血尿、腰腹痛。其中4例B超示肾占位性病变,1例示肾盂占位性病变。CT增强后强化不均匀。其中1例入院时双肺多发结节,其余4例人院时均未发现转移。2例行开放性根治性肾切除术,3例行肾切除术,术后病理报告为肾集合管癌伴乳头状结构。1例免疫组化示CK7阳性、vimentin部分阳性、CK20阴性,其余4例均无免疫组化结果。3例分别于术后9、12和15个月死于肾癌转移,2例无瘤生存。3例平均生存时间12．3个月。结论肾集合管癌临床症状明显,侵袭力强,预后差。目前术前诊断主要依赖CT和B超检查;主要治疗方法为根治性肾切除术,术后靶向治疗可能改善患者预后,但仍需要临床试验进一步验证。     

肾癌肾部分切除术后局部复发行二次手术患者的临床特征分析

目的探讨肾癌肾部分切除术后局部复发行二次手术患者的临床特征。方法回顾性分析2000年1月至2022年1月海军军医大学第一附属医院和海军军医大学第二附属医院收治的14例肾部分切除术后肾癌局部复发行二次手术患者的临床资料。男12例, 女2例;首次手术年龄(57.1±13.2)岁。14例中9例体质量指数≥24 kg/m2;初诊肿瘤R.E.N.A.L.评分≥7分者9例。首次手术均行肾部分切除术, 其中开放手术4例, 腹腔镜手术6例, 机器人辅助腹腔镜手术4例。首次手术病理pT1aN0M0期9例, pT1bN0M0期5例;透明细胞癌12例, 肉瘤样癌1例, 嫌色细胞癌1例;手术切缘未见肿瘤细胞。初次手术至局部复发的时间为(29.3±16.8)个月, 其中11例复发时间≥12个月, 7例复发时间>36个月。所有复发灶均为腹部CT或MRI检查发现, 13例复发时无临床症状。所有患者复发灶位置明确, 未见复发灶侵犯周围器官、组织征象, 无其他可疑病灶, 考虑肿瘤可完整切除。14例均行二次手术, 其中10例行根治性肾切除术[肿瘤最大径为(2.8±0.9)cm)];4例行肾部分切除术[肿瘤最大径为(...     

肾嫌色细胞癌的诊治（附4例报告）

目的 提高肾嫌色细胞癌的诊治水平。方法 回顾性分析4例肾嫌色细胞癌患者的临床资料。男3例，女1例，年龄34—76岁，平均52岁。结果 3例患者行肾癌根治术，1例行肾部分切除术。术后病理诊断为肾嫌色细胞癌，病理分期：pT1aNxMo 1例，pT1bNoMo3例。病理分级：G1 3例，G2 1例。免疫组织化学染色：CK8（低分子量细胞角蛋白）阳性，Vimentin（波型蛋白）阴性，Hale胶体铁阳性。随访4个月至8年，平均4年。4例患者均健在，无肿瘤复发或转移。结论 肾嫌色细胞癌是一种低度恶性的肾细胞癌，影像学检查对其诊断有重要帮助，确诊有赖于组织病理学检查，手术治疗后预后较好。     

乳头状肾细胞癌的临床特征分析（附7例报告）

目的：观察乳头状肾细胞癌的临床特点,提高对其诊断要点、治疗及预后的认识。方法：回顾性分析7例乳头状肾细胞癌患者的临床资料,复习相关文献,并对患者进行随访。结果：7例患者均经病理证实为乳头状肾细胞癌,1例并发肾上腺腺瘤;首发症状主要以腰痛、血尿、消瘦、低热为主;CT影像均提示肿瘤密度在肾髓质期强化程度明显弱于肾皮质,且在肾髓质期和排泄期呈均匀强化;6例经腹行根治性肾切除,1例经腰行根治性肾切除,术后均辅以免疫治疗,未行放化疗;6例获随访,随访时间为3个月～2年,1例于术后6个月出现急性肾衰死亡,1例于12个月因肝及淋巴结转移死亡,其余4例在随访期间未出现复发和转移,无瘤生存至今。结论：乳头状肾细胞癌与其它肾细胞癌在临床表现上基本相同,但在影像学表现,病理形态及生物学行为上均与其他类型的肾细胞癌不同,诊断主要依靠CT影像,确诊有赖于病理和免疫组织化学检查,早期手术是首选治疗方式,其预后可能与分期及转移有关。     

不同肾癌亚型组织中c-kit与增殖细胞核抗原的表达及意义

目的 探讨不同亚型肾癌组织中c-kit及增殖细胞核抗原(PCNA)的表达及临床意义.方法 采用免疫组化SP法测定137例透明细胞癌、82例乳头状癌、51例嫌色细胞癌标本中c-kit蛋白和PCNA的表达情况. 结果 嫌色细胞癌中c-kit强阳性表达率为94.1%(48/51),明显高于透明细胞癌的16.1%(22/137)及乳头状癌的28.1%(23/82),差异有统计学意义(P=0.001).c-kit强阳性表达仅与嫌色细胞癌分期有关(P=0.038);在透明细胞癌和乳头状癌中PCNA表达与分级关系密切(P=0.043,P=0.011),而与嫌色细胞癌分级无关. 结论 嫌色细胞癌中c-kit表达率明显高于其他肾癌亚型,且与肿瘤局部进展关系密切.不同亚型间c-kit表达差异有助于肾癌的临床鉴别诊断和指导晚期肾癌靶向治疗药物的选择.     

Nucleolar grade but not Fuhrman grade is applicable to papillary renal cell carcinoma

This study was undertaken to determine the validity of Fuhrman grading in a series of papillary renal cell carcinomas (PRCCs), to examine the interrelationship and prognostic significance of the individual components of the grading system, and further to determine whether any observed predictive value was independent of other prognostic indicators. Ninety cases of PRCC were studied. Fifty-nine tumors were of type 1 and 31 were of type 2. There were 33 TNM stage 1, 26 stage 2, 18 stage 3, and 12 stage 4 tumors, whereas division of cases according to pT category showed 14 pT1a, 20 pT1b, 25 pT2, 15 pT3a, 4 pT3b, and 11 pT4 tumors. Ten tumors were grade 1, 58 grade 2, and 22 grade 3 when predominant Fuhrman grade was assigned, whereas grading according to the high-power field containing the highest grade (focal grade) showed 40 grade 2, 49 grade 3, and 1 grade 4 tumors. Measurements of nuclear size (area, major axis, perimeter) and shape (shape factor, compactness) were undertaken using image analysis. Nuclear area ranged from 27.63 to 116.39 microM, major axis length 6.70 to 14.06 microM, and nuclear perimeter 20.05 to 41.77 microM. Shape factor ranged from 0.805 to 0.878 and compactness from 14.33 to 15.66. Predominant nucleolar grade using the criteria of the Fuhrman classification was nucleolar grade 1 for 13 tumors, nucleolar grade 2 for 56 tumors, and nucleolar grade 3 for 21 tumors. Focal nucleolar grade based on the high-power field showing the greatest degree of nuclear pleomorphism, was grade 2 for 38 tumors and grade 3 for 52 tumors. pT category, TNM stage, focal Fuhrman grade, and PRCC type were significantly associated with survival. Of the various measures of the components of the Fuhrman classification, only focal nucleolar grade was associated with survival, on univariate analysis. On multivariate analysis, focal nucleolar grade and tumor diameter were independently associated with survival, whereas TNM stage retained significance independent of other parameters. It is concluded that assessment of nucleolar prominence rather than Fuhrman grade is applicable for stratification of tumors within TNM stage or pT category for PRCC and that this should be based upon the high-power field showing the greatest degree of nuclear pleomorphism.     

Association of EMMPRIN and fascin expression in renal cell carcinoma: correlation with clinicopathological parameters

EMMPRIN and fascin are important factors in tumor invasion and progression. We tested the hypothesis that expression of EMMPRIN and fascin correlate with clinicopathological parameters of renal cell carcinoma (RCC). Immunohistochemical analysis of EMMPRIN and fascin were performed in tissue microarrays of 100 surgical specimens, including 35 clear-cell RCC (CRCC), 21 clear-cell RCC with granular differentiation (GRCC), 12 chromophobe RCC (ChRCC), 8 papillary RCC (PRCC), 9 carcinoma of the collecting duct of Bellini (CDC), 10 clear-cell RCC with sarcomatoid differentiation (SRCC), and 6 metastatic RCC. Average immunoscores of EMMPRIN were 100.8 in CRCC, 195.2 in GRCC, 298.4 in ChRCC, 219.2 in PRCC, 186.1 in CDC, 226.9 in SRCC, and 151.7 in metastatic RCC. Among all included cases, average EMMPRIN immunoscores were 84.6 in grade I, 130.4 in grade II, 184.3 in grade III, and 223.5 in grade IV. Additionally, average immunostaining scores of fascin were 53.6 in CRCC, 289.3 in GRCC, 193.3 in ChRCC, 151.8 in PRCC, 181.3 in CDC, 275.4 in SRCC, and 131.7 in metastatic RCC. Average fascin immunoscores were 59.3 in grade I, 91.6 in grade II, 130.2 in grade III, and 194.7 in grade IV. Higher EMMPRIN and fascin immunoscores also correlated significantly with TNM stages and survival rates in RCC. Significant correlation was found between EMMPRIN and fascin expression. In conclusion, higher expression of EMMPRIN and fascin correlate significantly with histological grades, clinical stages, and survival rates of RCC     

Do clear cell papillary renal cell carcinomas have malignant potential? Diolombi ML,Cheng L,Argani P,Epstein JI.Am J Surg Pathol. December 2015;39(12):1621–1634.

There have been no recurrences or metastases of clear cell papillary renal cell carcinoma (CCPRCC) in 268 reported cases with follow-up in the English-language literature. We identified all our cases of CCPRCC (1990–2013), reviewing all cases that preceded the formal designation of the entity. Immunohistochemical stains were performed on 32 cases during their initial workup. In addition, stains for carbonic anhydrase IX and cytokeratin 7 were performed on 2 cases, one with atypical follow-up and the other with a more compact morphology, although not performed initially. An extended panel with AMACR, CD10, and renal cell carcinoma (RCC) was added to the case with atypical follow-up. Fluorescence in situ hybridization for chromosomes 3p, 7, and 17 was performed on the latter case and on another clinically presumed metastatic tumor. In classic cases, immunohistochemical staining was not performed. Fifty-eight patients (31 women; 27 men) with follow-up data were included in our study; 39 cases were from our consult service. The patients? ages ranged from 36 to 83 years. Thirty-five patients had cystic or partially cystic lesions; 6 tumors were multifocal, 3 of which were bilateral. The majority (53 patients; 91.4%) presented with stage pT1 disease (size range: 0.2–8 cm), 2 patients presented with pT2 disease (8.5 and 10.3 cm), 1 patient presented with pT3 disease (6.5 cm sarcomatoid RCC focally extending out of the kidney), and pathologic stage was unavailable in 2 cases. Treatment consisted of 29 partial nephrectomies, 26 radical nephrectomies, 2 cryoablations, and 1 cyst ablation. The resection margins were negative in all but one case, with this case disease free after a 26-month period. Two patients had intraoperative tumor disruption and were disease free at 9 and 34 months. Five patients had synchronous ipsilateral renal cell carcinomas (non-CCPRCC). Mean follow-up time was 21 months (range: 1–175 months), with all but 3 patients having no evidence of disease. One patient was presumed to have contralateral disease on the basis of imaging findings and is alive and well 37 months after multiple partial nephrectomies. Metastatic disease to the lung was clinically presumed in 1 patient in whom a higher-grade lesion may have been missed during sampling of the predominantly cystic pT1b tumor and tissue confirmation of the metastases was not obtained. Another case presented with multiple skeletal and pulmonary metastases 8 months after resection of pT3 sarcomatoid CCPRCC. The patient with the sarcomatoid RCC died of multifocal skeletal and pulmonary metastatic disease 13 months after resection of the renal tumor. Our study, the largest to date with follow-up, along with others, suggests that pure CCPRCC is an indolent tumor and should be renamed “clear cell papillary neoplasm of low malignant potential” to reflect their biology.     

Rupture of papillary renal cell carcinoma

OBJECTIVE: Typical signs of papillary renal cell carcinoma (PRCC) are extensive necroses of the tumorous mass, which can modify the clinical appearance of PRCC. These necroses can imitate cysts on radiological examinations (ultrasonography and CT). The tumours are fragile and vulnerable to spontaneous rupture or rupture following minimal trauma (i.e. they act as a locus minoris resistentiae). MATERIAL AND METHODS: A total of 650 patients with a total of 671 renal tumours were surgically treated at our hospital between January 1991 and December 2003. RESULTS: In 16 cases bilateral tumours were found (in all cases RCC) and in five cases two types of tumour were identified in one kidney [all were a combination of conventional RCC (CRCC) and PRCC]. Altogether, 621 tumours (92.5%) were diagnosed as RCCs. Of these, CRCC was found in 563 cases (90.7%), PRCC in 36 (5.8%), chromophobe RCC in 14 (2.3%) and unclassified RCC in 7 (1.1%). All cases of ruptured PRCC were included in our study. Interestingly, only PRCCs ruptured in this series. Rupture was described in three cases of PRCC (8.3%): it was spontaneous in two cases and resulted from a traffic accident in the third. CONCLUSIONS: The extensive necrosis regularly found in PRCC can cause rupture of the tumour followed by retroperitoneal bleeding. Rupture affected <10% of our cases of PRCC. CT findings are usually not characteristic and can mimic a simple haematoma of unknown origin. Similarly, the perioperative finding is unclear in most cases. The final correct diagnosis of the renal tumour is frequently established only by the pathologist.     

上海仁济医院肾癌数据库资料分析

目的 探讨肾癌临床、病理、分期、分级与预后特征. 方法 分析2003年至2005年上海仁济医院泌尿科肾癌数据库435例患者临床和病理资料.采用WHO 1997年肾实质上皮性肿瘤组织学分类标准、2002年ATCC的TNM分期和临床分期、1982年Fuhrman病理分级.采用Kaplan-Meier法和Logrank检验对57例获随访的晚期患者行生存分析和预后因素判断. 结果 435例患者中,遗传性VHL病肾癌10例(2.4%)、散发性肾透明细胞癌372例(85.5%)、乳头状癌13例(3.0%)、嫌色细胞癌18例(4.1%)、集合管癌4例(0.9%)、嗜酸性细胞腺瘤4例(0.9 %)、未分类肾癌.14例(3.2%).行根治性肾切除术335例(77.0%),保留肾单位手术74例(17.0%),姑息性肾切除等手术26例(6.0%).遗传性VHL病肾癌均为双肾癌伴多发囊肿,临床分期Ⅰ期7例、Ⅱ期3例,病理分级Ⅰ级6例、Ⅱ级4例,基因测序均存在VHL基因突变,平均随访28.6个月,患者无肿瘤局部进展或转移,但4例患者出现同侧或双侧肿瘤再发.嫌色细胞癌临床分期均为Ⅰ期,病理分级Ⅰ级5例,Ⅱ级13例,平均随访19.8个月均存活,无肿瘤转移或复发.集合管癌临床分期均为Ⅰ期,病理分级均为Ⅲ级,平均生存时间11.3个月.肾透明细胞癌和乳头状癌临床分期Ⅰ期260例(67.6%)、Ⅱ期64例(16.6%)、Ⅲ期32例(8.3%)、Ⅳ期29例(7.5%),其中T1a 147例(38.2%)、T1b 113例(29.4 %);病理分级Ⅰ级124例(32.2%)、Ⅱ级219例(56.9%)、Ⅲ级40例(10.4%)、Ⅳ级2例(0.5%).57例晚期肾癌患者中位生存时间(16.0±1.3)个月,1年生存率55.0%,2年生存率31.0%.预后因素分析显示,临床分期、肿瘤大小、淋巴结转移、远处转移和病理分级是晚期肾癌解剖水平和组织学水平的预后影响因素. 结论 不同组织学亚型的肾癌生物学特征存在较大差异,遗传性VHL病肾癌存在基因突变,常为双侧、多中心、低Fuhrman分级透明细胞癌,易再发不易转移.肾嫌色细胞癌预后较好,而集合管癌预后差.在解剖水平和组织学水平,TNM分期、肿瘤大小、淋巴结转移、远处转移和肾癌病理分级是晚期肾癌的预后影响因素.     

肾癌的病理类型与预后的关系

目的探讨肾癌的病理类型与预后的关系。方法对315例肾癌患者根据病情选择相应的手术治疗和病理分型,并进行病例随访。以Kaplan-Meier法计算生存率。Cox回归模型对预后影响因子进行分析。结果其中透明细胞癌202例(71.9%),颗粒细胞癌51例(18.1%),混合性腺癌15例(5.3%),乳头状腺癌7例(2.5%),集合管癌4例(1.4%),肉瘤样肾癌2例(0.7%)。Cox回归模型多因素分析显示病理类型可能是一个独立的影响预后的因子。透明细胞癌、颗粒细胞癌和混合性腺癌患者的3年、5年生存率差别无统计学意义。7例乳头状腺癌仅1例死亡。4例集合管癌和2例肉瘤样癌均已死亡,两者平均存活时间分别为6.3和5.5月。结论肾癌的病理分型对预后有一定的预测价值;乳头状腺癌预后优于其他类型肾癌;集合管癌和肉瘤样癌预后较差。     

Pediatric renal cell carcinoma: single institution 25-year case series and initial experience with partial nephrectomy

PURPOSE: RCC represents less than 2% to 6% of pediatric renal tumors. Few reports of long-term outcomes exist. We sought to determine the presentation, treatment and outcome of patients at our institution. MATERIALS AND METHODS: We retrospectively reviewed the age, mode of presentation, mode of treatment, histological subtype, tumor grade, stage and survival of all patients with RCC from 1980 to 2005. RESULTS: A total of 15 patients were identified. Mean age at presentation was 7.9 years. Symptomatic presentations in nearly 75% of patients included gross hematuria, abdominal pain and polycythemia. The remaining 25% of cases were asymptomatic, and were identified by physical examination or incidentally on imaging. Surgical resection consisted of radical nephrectomy in 10 patients and partial nephrectomy in 5. Pathological analysis revealed papillary RCC in 8 patients and clear cell RCC in 7. Six patients had high stage disease. One patient with stage IV disease died 8 months postoperatively. The remaining 14 patients were alive at a mean followup of 4.9 years. All but 1 patient remain recurrence-free, including all of those who underwent nephron sparing surgery. CONCLUSIONS: To our knowledge this single institution series is the first to include children treated with partial nephrectomy. Pediatric patients with RCC tend to be older and more likely to present symptomatically compared to the typical patient with Wilms tumor. Hematuria and abdominal pain were the most common presentations, and papillary RCC was proportionately more common in this series. Our initial experience suggests that equivalent cure rates can be expected from a nephron sparing approach in appropriately selected cases.